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Distinct origins of the external carotid artery and the internal carotid artery (ICA) from the aortic arch have been rarely described, and represent an aberrant development of the aortic arches during fetal life. This anatomical variation is usually discovered incidentally; infrequently, an aneurysm of the cervical ICA might accompany this rare configuration. We describe one such case in a patient with Noonan syndrome who presented with pulsatile neck mass. The diagnostic features and management of the aneurysm and a review of the literature are presented.